DESCRIPTION (Adapted from applicant's description): The objective of this project is the cloning of the gene encoding mitochondrial carnitine/acylcarnitine translocase. The new set of Specific Aims include: 1) cloning and sequencing of the human CAC, cDNA and structural gene, 2) sequencing of CAC deficiency mutant genes, 3) examination of the expression of the mutant genes in mutant fibroblast cell lines, 4) creation of mutants in a yeast gene, yOR100c, that they believe to be yeast CAC and assessment of its genetic function, and 5) assaying CAC activity in mammalian cells in which mutant and wild-type CAC has been introduced.